Lipomatous Angiomyofibroblastoma in the Vulva

This is a case of a 55-year-old female, surgical menopause for nine years, not on hormone therapy with one year history of a progressively enlarging left vulvar mass, who sought consult and subsequently underwent excision of mass. Microscopic findings showed alternating hypo- and hypercellular areas composed of plump spindle to epithelioid cells distributed in an edematous stroma with varying amount of collagen. Tumor cells have bland chromatin, inconspicuous nucleoli, and absent mitoses. The cells surround small to medium-sized thin-walled, hyalinized vessels found in hypercellular areas. An adipocytic differentiation of more than fifty percent of tumor is observed further classifying this neoplasm as lipomatous angiomyofibroblastoma, making this an even rarer type of benign mesenchymal tumor of the vulva. Immunohistochemistry stains performed showed positive staining for vimentin, smooth muscle actin, BCL-2, ER, PR, and negative staining for desmin which supports the diagnosis. The clinical presentation, operative findings, histopathologic features and the various considerations are discussed. Literature review of vulvar angiomyofibroblastoma is also presented.